Introduction
Hereditary spherocytosis (HS) is a hemolytic anemia of varying severity caused by defects in the red blood cell membrane. Defects in the erythrocyte membrane and selective destruction of defective erythrocytes by the spleen are two key factors in the pathophysiological process of HS. The destruction of the red blood cells by the spleen can lead to proliferative enlargement of the spleen, which leads to increased red blood cell accumulation and/or increased hemolysis, thus creating a vicious cycle1.
The treatment of HS is aimed at minimizing the complications of chronic hemolysis and anemia. In addition to symptomatic treatment and blood transfusion, current guidelines recommend splenectomy if the patient has severe anemia complications or is transfusion dependent1–3. Since the spleen is a lymphoid organ, the overwhelming post-splenectomy infection (OPSI) is the most serious postoperative complication, especially in children. In addition to surgical risks, splenic resection is also prone to serious surgical complications such as venous thromboembolism (VTE), pulmonary arthritis, and pulmonary artery hypertension (PAH) after splenectomy4. Patients who have indications for splenectomy but were unwilling to undergo surgery, or who have contraindications to surgery, have been reported to be treated with PSE, which exhibited better outcomes and fewer surgical complications due to residual partial splenic immunity5,6. However, PSE had not been widely studied and applied in pediatric HS patients due to the poor controllability of the embolic range.
The aim of our study was to investigate another surgical procedure, which can achieve an accurate prediction of the extent of splenic embolism. We know that the splenic artery near the splenic hilum is usually divided into two branches at the upper and lower ends, each of which is further divided into 4-6 segmental intrapleural branches, with few traffic branches between these different segments. Thus, we first proposed SPSE to treat HS patients based on the anatomical characteristics of the splenic artery. The present study reports on the feasibility and efficacy of SPSE in a cohort of 16 children with HS.